Edwin L. Steele Laboratories, Department of Radiation Oncology, Massachusetts General Hospital and Harvard Medical School, Boston, MA 02114, USA.
Eaton-Peabody Laboratories and Department of Otolaryngology, Head and Neck Surgery, Massachusetts Eye and Ear and Harvard Medical School, Boston, MA 02114, USA.
Sci Transl Med. 2021 Jul 14;13(602). doi: 10.1126/scitranslmed.abd4816.
Hearing loss is one of the most common symptoms of neurofibromatosis type 2 (NF2) caused by vestibular schwannomas (VSs). Fibrosis in the VS tumor microenvironment (TME) is associated with hearing loss in patients with NF2. We hypothesized that reducing the fibrosis using losartan, an FDA-approved antihypertensive drug that blocks fibrotic and inflammatory signaling, could improve hearing. Using NF2 mouse models, we found that losartan treatment normalized the TME by (i) reducing neuroinflammatory IL-6/STAT3 signaling and preventing hearing loss, (ii) normalizing tumor vasculature and alleviating neuro-edema, and (iii) increasing oxygen delivery and enhancing efficacy of radiation therapy. In preparation to translate these exciting findings into the clinic, we used patient samples and data and demonstrated that IL-6/STAT3 signaling inversely associated with hearing function, that elevated production of tumor-derived IL-6 was associated with reduced viability of cochlear sensory cells and neurons in ex vivo organotypic cochlear cultures, and that patients receiving angiotensin receptor blockers have no progression in VS-induced hearing loss compared with patients on other or no antihypertensives based on a retrospective analysis of patients with VS and hypertension. Our study provides the rationale and critical data for a prospective clinical trial of losartan in patients with VS.
听力损失是神经纤维瘤病 2 型(NF2)的最常见症状之一,由前庭神经鞘瘤(VS)引起。VS 肿瘤微环境(TME)中的纤维化与 NF2 患者的听力损失有关。我们假设使用洛沙坦(一种 FDA 批准的抗高血压药物,可阻断纤维化和炎症信号)减少纤维化可以改善听力。使用 NF2 小鼠模型,我们发现洛沙坦治疗通过以下方式使 TME 正常化:(i)减少神经炎症性 IL-6/STAT3 信号并预防听力损失;(ii)使肿瘤血管正常化并减轻神经水肿;(iii)增加氧气输送并增强放射治疗的效果。为了将这些令人兴奋的发现转化为临床实践,我们使用患者样本和数据证明,IL-6/STAT3 信号与听力功能呈负相关,肿瘤衍生的 IL-6 产量升高与体外器官型耳蜗培养物中的耳蜗感觉细胞和神经元活力降低有关,并且根据对 VS 和高血压患者的回顾性分析,与接受血管紧张素受体阻滞剂的患者相比,接受其他降压药或无降压药的患者 VS 引起的听力损失没有进展。我们的研究为洛沙坦在 VS 患者中的前瞻性临床试验提供了依据和关键数据。